Diagnosis

When the diagnosis of ILD is suspected, your doctor considers the following:

Your medical history – This includes: a review of all the person’s symptoms, a listing of current and former medications and a review of any environmental and occupational exposures to dust, gases, chemicals, pets (in particular birds) and humidifiers.

A complete physical examination - Findings of crackling sounds in the lungs and changes in the skin, joints and fingernails can help direct further evaluation.

A chest X-ray and high resolution computed tomography (CT) scan - Results are often abnormal with ILD. This can often help determine the type of ILD present.

Pulmonary function tests - These breathing test results are often abnormal with ILD. Your lung function may be checked before and after an inhaled bronchodilator treatment.

Arterial blood gas - The arterial blood gas test measures the amount of oxygen and carbon dioxide in your blood. The results may be normal or show a reduced oxygen level.

Exercise testing - Exercise tests may show a decrease in your oxygen level during activity. Testing may include a six minute walk, oxygen titration and exercise tolerance test.

Laboratory data – Blood work may be done to test for autoimmune disease and other disorders that can cause ILD.

Bronchoscopy with bronchoalveolar lavage – A bronchoscopy may be done to check for inflammatory cells in your lungs or to sample small pieces of the lung to look for evidence of a specific ILD. Bronchoscopy involves inserting a tube through the nose into your trachea (windpipe) to see the airways. In bronchoalveolar lavage a small amount of sterile saline is placed in one area of your lung and then withdrawn. This fluid contains cells that will be analyzed under the microscope. Small biopsy tools may be used through the bronchoscope if indicated.

Surgical lung biopsy – In many cases of ILD, a surgical lung biopsy is needed to get enough lung tissue to make a specific diagnosis. This may be performed with the use of a thoracoscope. This allows the surgeon to biopsy multiple areas of one lung through a few very small incisions.

Clinical Trials

A Linkage and Association Study in Pulmonary Fibrosis

The purpose of this study is to investigate inherited genetic factors that play a role in the development of familial pulmonary fibrosis and to identify a group of genes that predispose individuals to develop pulmonary fibrosis. Finding the genes which play a role in pulmonary fibrosis is the first step at developing better methods for early diagnosis and improved treatment for pulmonary fibrosis. The overall hypothesis is that inherited genetic factors predispose individuals to develop pulmonary fibrosis.

The study requires the following:

For affected individuals, we confirm your diagnosis by reviewing your medical records. We also ask for a history questionnaire to be filled out and obtain a sample of blood or tissue for genetic studies.

Unaffected family members are screened with a questionnaire, and a blood sample is obtained for genetic studies. Some family members may qualify to undergo high-resolution CT (HRCT) scan for the study.

To be eligible:

The only requirement for this study is to have at least 2 members of your family diagnosed with Idiopathic Pulmonary Fibrosis (IPF). You may be included in this number.